RECURRENT DIFFUSE ALVEOLAR HEMORRHAGE IN SYSTEMIC SCLEROSIS AS COMPLICATION OF COVID-19

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Diffuse alveolar hemorrhage (DAH) is a rare but severe life-threatening condition characterized by widespread bleeding of the pulmonary vasculature into the alveolar space. DAH have a myriad of underlying etiologies. Rheumatic diseases have shown association with DAH. The Coronavirus disease 2019 (COVID-19) pandemic has increased the complexity associated with diagnosing DAH. Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) infects the endothelium using the angiotensin-converting enzyme 2 receptor causing a lymphocytic endotheliitis. The increased inflammatory state secondary to COVID-19 has been associated with development of vasculitis syndromes. Although systemic sclerosis (SSc) has been linked to recurrent DAH, there is scarce evidence associating COVID-19 and SSc with DAH. CASE PRESENTATION: A 61-year-old female with a past medical history of SSc, interstitial lung disease (ILD) and DAH presented with worsening shortness of breath of 2 days duration. She was found to have acute hypoxic respiratory failure. Computed tomography (CT) of thorax showed diffuse bilateral ground-glass opacities. SARS-Cov-2 polymerase chain reaction was positive. She was started on broad spectrum antibiotics, dexamethasone and received two units of COVID-19 convalescent plasma. Due to respiratory distress, she was intubated. A decline in her hemoglobin prompted a diagnostic bronchoscopy that showed increasing frank blood in the bronchoalveolar lavage. Antinuclear antibody with centromere pattern and rheumatoid factor were positive with low complement C3 and C4. Steroids were switched to high-dose IV methylprednisolone. Three cycles of plasmapheresis were given with no clinical improvement. DISCUSSION: COVID-19 pandemic has changed the traditional approach to dyspnea. This change in the paradigm, as a result of limited resources and increased occupational risk, may hinder the diagnosis of complex disorders like SSc-ILD and DAH. It's of paramount importance to test rapidly for SARS-CoV-2 infection. ILD is a common complication of SSc. The high-resolution CT similarities between the 2 diseases make it difficult to distinguish COVID-19-ILD superinfection from worsening of SSc-ILD. Bronchoscopy and surgical lung biopsy should always be weighed against their possible complications, occupational exposure and prognosis. CONCLUSIONS: COVID-19 may overlap and complicate the diagnosis of SSc-ILD. COVID-19 associated acute respiratory distress syndrome is attributed to cytokines release (cytokine storm). This likely worsens the underlying inflammatory process in SSc-ILD and could explain the development of DAH. Although there is limited evidence to guide specific treatment, steroid therapy has proven to have mortality benefit in severe COVID-19. The role of steroids, plasmapheresis, immunomodulators and antifibrotic therapy in COVID-19 with SSc-ILD complicated with DAH is unknown. REFERENCE #1: Colby TV, Fukuoka J, Ewaskow SP, Helmers R, Leslie KO. Pathologic approach to pulmonary hemorrhage. Annals of Diagnostic Pathology. 2001;5(5):309-19. REFERENCE #2: Varga Z, Flammer AJ, Steiger P, Haberecker M, Andermatt R, Zinkernagel AS, et al. Endothelial cell infection and endotheliitis in COVID-19. The Lancet. 2020;395(10234):1417-8. REFERENCE #3: Wong AW, Fidler L, Marcoux V, Johannson KA, Assayag D, Fisher JH, et al. Practical Considerations for the Diagnosis and Treatment of Fibrotic Interstitial Lung Disease During the Coronavirus Disease 2019 Pandemic. Chest. 2020;158(3):1069-78. DISCLOSURES: No relevant relationships by Vedesh Babu, source=Web Response No relevant relationships by Alejandra Garcia Fernandez, source=Web Response No relevant relationships by ahmad hamdan, source=Web Response No relevant relationships by Carlos Matute Martinez, source=Web Response